Moyamoya in Hispanics: not Only in Japanese

Abstract

Moyamoya disease was first described in 1957 as hypoplasia of the bilateral internal carotid arteries, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is moyamoya. This paper describes two cases of moyamoya presentations, including moyamoya disease and moyamoya syndrome. Moyamoya may rarely occur in North American Hispanic patients. The presentation can vary significantly and ranges bwtween fulminant outcome and prolonged survival. Awareness about moyamoya and its different presentations may be beneficial for the patients and can improve the outcome.

Keywords: Hispanic population; moyamoya disease; moyamoya syndrome; stroke.

Figure 1.

Case #1. A) and B) diffusion weighted sequence and apparent diffusion coefficient map, respectively, demonstrate a focal area of restriction of diffusion involving the right rostrum, genu and body of the Corpus Callosum with mild enhancement on postgadolinium T1W sequence (C) and moderate edema and local mass effect on T2W sequence (D). A second lesion involving the right basal ganglia and genu of the right internal capsule demonstrates incomplete peripheral enhancement with central necrosis, without mass effect or restriction of diffusion.

Figure 2.

Case #1. Biplanar cerebral angiography of the left internal carotid artery (A and B, lateral and AP respectively) demonstrated complete occlusion of the left supraclinoid internal carotid artery with a patent left anterior choroidal artery supplying lenticulostriatal vessels giving the angiographic appearance of a I, suggestive of Moyamoya angiographic pattern. Collateral flow from the right posterior choroidal artery supplying the pericollosal, frontopolar and callosmarginal arteries demonstrated on lateral view on selective angiogram of the left vertebral artery (C). Intracranial view of the right internal carotid artery in the AP projection (D) demonstrates an irregular M1 segment and complete occlusion of the mid A1 segment of anterior cerebral artery. Cortical branches also appeared irregularity consistent with a proliferative intracranial vasculopathy.

Figure 3.

Case #2. Bilateral severe stenosis (near occlusion) of intracranial supraclinoid internal carotid arteries (left worse than right) with multiple transdural basilar collaterals consistent with moyamoya angiographic pattern.